Immunoglobulin G4 related disease (IgG4-RD) is a recently recognized distinct disease entity that can affect many organs/tissues. The kidney is a frequently involved organ with tubulointerstitial nephritis (TIN), and the renal lesions are collectively referred to as IgG4-related kidney disease (IgG4-RKD). Although definitive diagnosis requires histopathologic analysis, imaging plays a crucial role in demonstrating the involved organs/tissues. We retrospectively reviewed the magnetic resonance (MR) imaging findings of 13 patients diagnosed as IgG4-RKD. Various features were assessed, including renal size, margin between cortex and medulla, signal intensity on T1, T2 and diffusion weighted MR images, contrast enhancement, collecting system and/or renal fascia changes. MR imaging features, such as morphology changes, signal intensity abnormality especially on DWI, collecting system and/or renal fascia involvement enables the diagnosis of IgG4-RKD.
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